ReviewTreatment guidelines of lymphatic malformations of the head and neck
Section snippets
Clinical and histopathological features
Most lymphatic malformations appear at birth, but the clinical symptoms often present at older ages. Gross6 reported that 50–75% can be diagnosed at birth, with 80–90% of the remaining cases diagnosed by the age of 2 years. Infection, trauma or changes in hormone level can result in destruction of lymphatic circulation and expansion of the lesions. Radiation therapy or connective tissue diseases (CTDs) can also aggravate the condition.
More than 70% of lymphatic malformations are found in the
Diagnosis and treatment
It is not difficult to make the diagnosis of LMs based on history and clinical manifestations. Lesions in oral mucosa commonly present as microcystic, isolated or multiple sporadic round nodules or punctuate lesions. The lesion is soft in texture and pink to dark red in color scattered with yellow vesicles, sometimes with venous malformations. The most commonly affected areas of deep-seated LMs are the lower 2/3 of the face such as the lip, cheek, tongue and ear, etc., which often cause
Treatment modalities
Many treatment methods have been documented in the literature, including surgery, various sclerotherapy and laser therapy. Localized microcystic lesions can be resected completely, but impossible for extensive and diffuse ones, the reasons being: (1) most of them involve the lip, cheek and tongue, complete excision may result in severe tissue defects, leading to cosmetic and functional complications; (2) the lesions are poorly demarcated; (3) the walls of lymphatic vessels of LMs are thin and
Choice of treatment methods
The choice of treatment should be individualized and based on several factors such as the hyoid level, bilaterality, age of onset, growth rate, type, depth, extent, anatomical location, potential deformity or dysfunction of LMs.19 Lesions with severe life-threatening functional impairment should be treated early. When there is no significant functional deficit, treatment can be delayed well past infancy and 18–24 months is available to allow for spontaneous resolution.[20], [21] Localized
Intralesional injection of Pingyangmycin
Bleomycin is a cytotoxic antitumor antibiotic that can be administered intralesionally by means of transcutaneous injection and is proving to be an exciting modulator of vascular anomalies.22 Pingyangmycin (PYM) is a single-component A5 of various components of bleomycin, because of the low cost, safety, and ease of availability, percutaneous intralesional injection of Pingyangmycin has been used more frequently for management of vascular malformations as a single modality or in combination
Laser therapy
Laser therapy has been used to treat superficial lymphatic malformations, especially for patients with localized infection. The advantage of laser therapy is the ease of use, less bleeding, minimal pain, reliable effect and repeatable treatment. Atropine can be used preoperatively to reduce salivary secretion. The wavelength of CO2 laser is 10,600 nm (far-infrared light) with 0.2 mm spot size and 0–6 W continuous output power. The laser probe should be kept at a distance of 0.5–1 cm from the
Combined therapy
Laser therapy and PYM sclerotherapy can be used alternatively or jointly for superficial microcystic lymphatic malformations of the tongue and oral mucosa. The local tissues may become thicker and harder after several injections. Local injection of triamcinolone acetonide can loosen and soften the tissues making the lesions thinner. It must be addressed that the pharmaceutic effect of triamcinolone acetonide could last 2 months, thus the dosage for children below 2 years old should be less then
Deep-seated microcystic lymphatic malformations
Treatment of deep-seated microcystic LMs is still a challenging problem; surgical resection alone usually has a poor outcome, often resulting in secondary deformities. Therefore, surgery is no longer the preferred method for deep-seated microcystic LMs. Intralesional injection of OK-432 or Pingyangmycin has been recommended with excellent result in some patients through repeated injections. For complicated cases, surgical correction may be applied after laser therapy and/or sclerotherapy to
Macrocystic lymphatic malformations
Sclerotherapy with Pingyangmycin or OK-432 is the mainstay of treatment for macrocystic lymphatic malformations, and surgery is usually used as complementary therapy.[14], [27] The lymphatic fluid in the cystic space should be aspirated as much as possible through 7-gauge needle prior to injection of Pingyangmycin (2 mg/mL) or OK-432. The amount of OK-432 (0.1 mg/10 mL) is the same as the aspirated fluid, not more than 20 mL. Large macrocystic lymphatic malformations localized in the upper neck or
Conclusion
A number of treatment methods are available for lymphatic malformations of the head and neck region. Sclerotherapy has been most effective in the management of macrocystic LM. Superficial and localized lesions can be treated with intralesional injection and laser therapy, often with good results. If lesions are too extensive for complete excision, sclerotherapy may be used as the primary treatment of choice or postoperative adjunctive treatment. The use of laser therapy and sclerotherapy is
Conflict of interest statement
None declared.
Acknowledgment
We are very grateful to Dr. Kee Hau Wong from ParkwayHealth Shanghai for his careful revision of the manuscripts and valuable suggestions.
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